[Thymic and bronchial neuroendocrine tumors in multiple endocrine neoplasia type 1. GENEM1].
OBJECTIVE
Multiple endocrine neoplasia type 1 (MEN 1) mainly affects parathyroid glands, pancreatic islets and pituitary gland. The aim of this study in 95 MEN 1 patients was to examine less frequent localizations thymic and bronchic neuroendocrine tumors (NET).


METHODS
Two cases of bronchic NET were observed, both in women, and 4 cases of thymic NET, all in men, giving a prevalence of 7.3%. These NET were often asymptomatic. A metastatic diffusion was observed in 3 cases. Elevated plasma levels of glycoprotein hormone alpha subunit (SU) and FSH were observed in 3 and 1 cases respectively. Immunohistochemistry indicated the tumor cells to be frequently positively stained for HCG alpha and FSH.


CONCLUSIONS
We conclude that all patients with MEN 1 should undergo screening for thoracic NET, especially in high risk familial subgroups and in case of elevated plasma alpha SU or FSH.
Murat
A
A
Clinique d'Endocrinologie, Hôtel-Dieu, Nantes.
Heymann
M F
MF
Bernat
S
S
Dupas
B
B
Delajartre
A Y
AY
Calender
A
A
Despins
P
P
Michaud
J L
JL
Giraud
S
S
Le Bodic
M F
MF
Charbonnel
B
B
D000328:Adult; D001984:Bronchial Neoplasms / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000276:immunology / Q000503:physiopathology*; D011446:Prospective Studies; D012189:Retrospective Studies; D013953:Thymus Neoplasms / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology*
Tumeurs neuro-endocrines thymiques et bronchiques au cours des néoplasies endocrines multiples de type 1. GENEM1.